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Summary A 44-year-old woman with seronegative polyarthritis presented with a 2-year history of a solitary, bluish-red, oedematous, nonscaly, annular and partially reticulated macule on her right thigh. Histopathological findings revealed perivascular and periadnexal lymphocytic infiltrate in the dermis. Alcian blue and colloidal iron stains highlighted mucinous deposit in the upper and mid dermis. Direct immunofluorescence showed a linear deposit of IgG and C3 along the basement membrane zone. Antinuclear antibody was positive at a titre of 1 : 80, with homogenous and speckled patterns. Except for its unusual localization and lack of photosensitivity, our case had the clinical and histopathological features of lupus erythematosus tumidus. These characteristics were also reminiscent of reticular erythematous mucinosis and erythema annulare centrifugum, both of which are considered to be associated with cutaneous lupus erythematosus (CLE). Hydroxychloroquine 200 mg daily led to improvement of the skin lesion. The unusual clinical presentation of our case emphasizes the heterogeneity of clinical manifestations of CLE.

The nomenclature and classification of cutaneous mucinosis is quite complex. An updated classification of idiopathic cutaneous mucinosis (lichen myxoedematosus), included three clinicopathological subsets: a generalized papular and sclerodermoid form, a localized papular form, and an atypical or intermediate form. Cutaneous mucinosis occurring in infancy is very rare. We report a 7-month-old boy with a history of multiple opalescent papules over his fingers and toes since birth. The lesions spontaneously regressed and became indiscernible after 5 years of clinical follow-up. The condition was similar to cutaneous mucinosis of infancy but was also unique because of its spontaneous regression and acral location.

Summary Background Lymphocytic infiltration of the skin (LIS) and reticular erythematous mucinosis (REM) are characterized histologically by an inflammatory cutaneous lymphocytic infiltrate similar to the histological appearance of pseudolymphoma. Objectives To re-evaluate a large cohort of patients with the clinical and/or histological diagnosis or differential diagnosis of LIS and REM and to assess the evidence for infection with Borrelia. Methods Sixty-nine cases of LIS and 34 cases of REM were retrospectively investigated. Haematoxylin and eosin sections were re-examined, and histological diagnoses were specified and confirmed by clinicopathological correlation. Evidence for Borrelia infection was assessed by immunohistochemistry and focus-floating microscopy (FFM). Results LIS appeared to serve as a collective term for two main clinicopathological reaction patterns: (i) (tumid) lupus erythematosus (LE) (32 of 69, 46%) and (ii) pseudolymphoma (31 of 69, 45%). Other diagnoses (five of 69, 7%) included polymorphic light eruption, arthropod bite reaction, spongiotic dermatitis, drug eruption and urticaria. Spirochaetes were detected by FFM in 24 of 31 (77%) cases with a pseudolymphomatous reaction, while all nonpseudolymphomatous reactions were negative. Of the cases initially considered as REM, 21 of 34 (62%) were classified as LE, four of 34 (12%) as pseudolymphoma (three of four positive for Borrelia), and five of 34 (15%) as other diagnoses (folliculitis, morphoea, seborrhoeic dermatitis, prurigo and arthropod bite reaction). The diagnosis of Borrelia-associated pseudolymphoma was made significantly more often in those cases where LIS was considered as initial differential diagnosis than REM (P

Cutaneous diseases are often found in obese patients but, to our knowledge, mucinous disorders have not been previously reported in association with obesity. Two cases of localized lichen myxoedematosus (papular mucinosis) in two women with morbid obesity are described. Both patients underwent a low-calorie diet for a 1-year period in one case, and for 4 months in the other one, as the only treatment. There was complete resolution of cutaneous lesions at the same time that an important weight loss was observed. Nevertheless, although spontaneous regression is not frequent, it could not be disregarded in either of these two cases.

We report a patient with reticular erythematous mucinosis (REM) syndrome. Content of hyaluronan in lesional skin was approximately 2.9-fold higher than in the patient's uninvolved skin, but its synthetic activity in fibroblasts explanted from lesional skin remained unchanged. Immunohistochemical study using antifactor XIIIa (anti-FXIIIa) antibody demonstrated that the number of FXIIIa+ cells in the lesional skin was significantly increased compared with those in the patient's uninvolved skin and in normal control skin samples (P < 0.01). As hyaluronan is considered to be synthesized by hyaluronan synthase (HAS), which is composed of three genetically distinct isoforms (HAS1, HAS2 and HAS 3). the cells responsible for the accumulation of hyaluronan in lesional skin were immunohistochemically examined using antibodies for HAS1, HAS2 and HAS 3. The specific antibody for HAS2 was found to react with some populations of FXIIIa+ cells in the involved skin, and the number of HAS2+ cells was significantly increased in the involved skin (P < 0.01). The results suggest that accumulation of hyaluronan in REM may be related to populations of FXIIIa+/HAS2+ dermal dendrocytes rather than to dermal fibroblasts.

A 41-year-old white man is described with papules of the lower and upper back, the neck and the upper chest, a marked deposition of mucin in the upper reticular dermis, and an IgG lambda monoclonal gammopathy strongly evocative of scleromyxedema (lichen myxedematosus). Additionally, he developed intense myalgia, muscle weakness and rhabdomyolysis, which were associated with heliotrope erythema, photosensitivity and an erythematous rash of the dorsum of the hands with Gottron's papules. Muscle biopsy revealed an inflammatory myositis, and dermatomyositis was diagnosed. The association of dermatomyositis and secondary mucinosis, or muscle involvement in primary papular mucinosis are not rare. However, the association between scleromyxedema and dermatomyositis has only exceptionally been reported.

Secondary cutaneous mucinosis is a well-recognized feature of connective tissue diseases such as lupus erythematosus and dermatomyositis. We report the first three cases of dermal mucinosis in association with severe chronic cutaneous graft-versus-host disease of the sclerodermoid variety. One patient had clinical changes due to abundant mucin accumulation within the papillary dermis (mucinoma). In the other two patients histological examination revealed extensive deposits of mucin predominantly within the reticular dermis. The microscopic appearances were striking, with numerous vacuolated spaces interspersed between collagen bundles. We speculate that this appearance is the result of ground substance becoming trapped within grossly sclerodermoid connective tissue.

A 67-year-old white man is described who had large sclerotic plaques with a violaceous border on his back and abdomen and a scleroderma-like induration on the legs, sparing the feet. Additionally, he had multiple skin-coloured, large, sometimes centrally depressed papules and nodules on the nape of the neck, back and upper limbs. These occurred in both the normal and sclerotic areas. Histologically, large amounts of focal mucin were seen in the upper dermis consistent with the diagnosis of papular mucinosis and associated with typical underlying features of scleroderma. No paraproteinaemia was found. We excluded scleromyxoedema on clinical, histopathological and laboratory grounds and diagnosed our patient as having generalized morphoea with papular mucinosis. We are aware of only two reports of large cutaneous deposits of mucin associated with scleroderma.

We describe two women, aged 65 and 37 years, who presented with mucinous plaques as the initial skin signs of dermatomyositis. Other cutaneous manifestations were lacking, but typical myositis appeared later in the course of the disease.

Lichen myxoedematosus (LM), or papular mucinosis, is an uncommon papular eruption caused by the dermal deposition of mucin. Three clinical forms can be distinguished, namely localised, disseminated (involving more than one site), and generalised LM, the last is called scleromyxoedema, and demonstrates erythema and skin sclerosis. Paraproteinaemia, often consisting of an abnormal IgG with lambda light chains, is usually present in patients with LM. Visceral involvement has also been documented. An association between LM and human immunodeficiency virus (HIV) infection has been reported recently. We now describe two further HIV-positive patients with LM and present a review of the literature regarding this association.

We report a case with the clinical and histological features of the reticular erythematous mucinosis syndrome (REM), in which there was moderate, continuous, fine, granular, IgM deposition along the basal layer. Similar direct immunofluorescence results have been reported in only two previous cases.

Summary We describe two young sisters with an asymptomatic papular eruption on the forearms, the clinical, histopathological and ultrastructural features of which were consistent with acral persistent papular mucinosis. Familial occurence of this uncommon disease is exceptional.

Adult Still's disease (ASD) is a rare disorder of unknown aetiology, characterized by an evanescent, erythematous, maculopapular rash, fever, arthralgia, and a variety of systemic features. We report a case which illustrates the typical features of ASD, and manifests the hitherto unreported complication of diffuse cutaneous mucinosis.